Michael S Okun1. 1. University of Florida McKnight Brain Institute, Gainesville, Florida 32610, USA. okun@neurology.ufl.edu
Abstract
BACKGROUND: Since the first descriptions of chorea in the Middle Ages, medicine has been captivated by the mysterious appearance of patients suffering from contortions of the body. Many innocent victims were persecuted and even tried as witches as the medical community struggled to arrive at a better understanding of Huntington's disease. DESIGN/ METHODS: The contributions, before and after Huntington's essay, that have led to a better understanding of adult onset hereditary chorea are examined in this paper. A review is undertaken of the American and international medical literature including articles, books, and journals. The impact of Huntington's original essay is discussed in detail. RESULTS: The history of adult onset chorea dates back to the Middle Ages when descriptions were vague, and sufferers were persecuted. Nineteenth century doctors recognized and reported the distinct entity of adult onset hereditary chorea. Perhaps with the help of an expanded life expectancy, George Huntington described the autosomal dominant inheritance pattern as well as other characteristic features of the disease. Later, distinctive phenotypes and the juvenile form were reported. The modern history of the disease remained relatively quiescent until the discovery of the causative gene in 1983. This discovery marked a new era in the history of adult onset chorea. CONCLUSIONS: Accurate descriptions before and after Huntington's essay about hereditary chorea have provided humanity to sufferers and the nidus for new discovery within the field of movement disorders.
BACKGROUND: Since the first descriptions of chorea in the Middle Ages, medicine has been captivated by the mysterious appearance of patients suffering from contortions of the body. Many innocent victims were persecuted and even tried as witches as the medical community struggled to arrive at a better understanding of Huntington's disease. DESIGN/ METHODS: The contributions, before and after Huntington's essay, that have led to a better understanding of adult onset hereditary chorea are examined in this paper. A review is undertaken of the American and international medical literature including articles, books, and journals. The impact of Huntington's original essay is discussed in detail. RESULTS: The history of adult onset chorea dates back to the Middle Ages when descriptions were vague, and sufferers were persecuted. Nineteenth century doctors recognized and reported the distinct entity of adult onset hereditary chorea. Perhaps with the help of an expanded life expectancy, George Huntington described the autosomal dominant inheritance pattern as well as other characteristic features of the disease. Later, distinctive phenotypes and the juvenile form were reported. The modern history of the disease remained relatively quiescent until the discovery of the causative gene in 1983. This discovery marked a new era in the history of adult onset chorea. CONCLUSIONS: Accurate descriptions before and after Huntington's essay about hereditary chorea have provided humanity to sufferers and the nidus for new discovery within the field of movement disorders.